Reproducibility and Inter-rater Reliability of 2 Paediatric Nutritional Screening Tools.

OBJECTIVES: The aim of the present study was to assess reproducibility and inter-rater reliability of 2 nutritional screening tools (NST): Screening Tool for Risk on Nutritional Status and Growth (STRONGkids) and Screening Tool for the Assessment of Malnutrition in Paediatrics (STAMP). METHODS: Prospective observational multicentre study. Patients ages 1 month or older admitted to paediatric or surgical wards were tested within 24 hours of admission by 2 independent observers: experts specialized in paediatric nutrition (physicians or dieticians) and clinical staff nonexpert in nutrition. Diagnosis on admission, underlying diseases, and length of stay were registered. STATISTICAL ANALYSIS: Kappa index (κ) to evaluate agreement between observers. RESULTS: A total of 223 patients were included (53.4% boys), with mean age of 5.59 (95% confidence interval 4.94-6.22) years. Experts classified 9.9% of patients at high risk with STRONGkids and 19.7% using STAMP, whereas nonexpert staff assigned 6.7% of patients to the high-risk category with STRONGkids and 21.9% with STAMP. Agreement between expert and nonexpert staff was good: 94.78% for STRONGkids (κ 0.72 [P < 0.001]); 92.55% for STAMP (κ 0.74 [P < 0.001]). The rate of malnutrition was significantly higher among high-risk patients with both NST, independent of examiner experience. After adjusting for age, both STRONGkids and STAMP high-risk scores predicted longer length of stay, whether assessed by experts or nonexperts, although differences were higher with STRONGkids. CONCLUSIONS: Agreement between experts and nonexpert staff in nutrition was good, producing a similar high-risk patient profile. Our results demonstrate that these NSTs are appropriate for nutritional screening in settings in which users have no previous experience in the field.

Urea cycle disorders in Spain: an observational, cross-sectional and multicentric study of 104 cases.

BACKGROUND: Advances in the diagnosis and treatment of urea cycle disorders (UCDs) have led to a higher survival rate. The purpose of this study is to describe the characteristics of patients with urea cycle disorders in Spain. METHODS: Observational, cross-sectional and multicenter study. Clinical, biochemical and genetic data were collected from patients with UCDs, treated in the metabolic diseases centers in Spain between February 2012 and February 2013, covering the entire Spanish population. Heterozygous mothers of patients with OTC deficiency were only included if they were on treatment due to being symptomatic or having biochemistry abnormalities. RESULTS: 104 patients from 98 families were included. Ornithine transcarbamylase deficiency was the most frequent condition (64.4%) (61.2% female) followed by type 1 citrullinemia (21.1%) and argininosuccinic aciduria (9.6%). Only 13 patients (12.5%) were diagnosed in a pre-symptomatic state. 63% of the cases presented with type intoxication encephalopathy. The median ammonia level at onset was 298 µmol/L (169-615). The genotype of 75 patients is known, with 18 new mutations having been described. During the data collection period four patients died, three of them in the early days of life. The median current age is 9.96 years (5.29-18), with 25 patients over 18 years of age. Anthropometric data, expressed as median and z-score for the Spanish population is shown. 52.5% of the cases present neurological sequelae, which have been linked to the type of disease, neonatal onset, hepatic failure at diagnosis and ammonia values at diagnosis. 93 patients are following a protein restrictive diet, 0.84 g/kg/day (0.67-1.10), 50 are receiving essential amino acid supplements, 0.25 g/kg/day (0.20-0.45), 58 arginine, 156 mg/kg/day (109-305) and 45 citrulline, 150 mg/kg/day (105-199). 65 patients are being treated with drugs: 4 with sodium benzoate, 50 with sodium phenylbutyrate, 10 with both drugs and 1 with carglumic acid. CONCLUSIONS: Studies like this make it possible to analyze the frequency, natural history and clinical practices in the area of rare diseases, with the purpose of knowing the needs of the patients and thus planning their care.

Detección precoz de la desnutrición y/o riesgo de desnutrición en niños / Early detection of malnutrition and risk of malnutrition in child

La desnutrición es frecuente en los niños hospitalizados, está asociada a un aumento de la estancia hospitalaria y a numerosas prescripciones, por tanto a un aumento del costo y a mayor número de infecciones. La desnutrición durante la infancia ha sido asociada con un pobre crecimiento, reducidas adquisiciones en la educación y conocimientos y posiblemente tenga implicaciones en la salud en la edad adulta. Para prevenir la malnutrición y la malnutrición adquirida en el hospital es necesario identificar precozmente a los pacientes desnutridos, incluso en el momento del ingreso, también se deberán identificar a los que tienen riesgo de desnutrirse durante la enfermedad, aunque estas recomendaciones no han podido incluirse en las rutinas de la practica clónica. La prevalencia de la malnutrición aguda hospitalaria en lactantes y niños va desde un 6,1% a un 32% dependiendo de la edad, del tipo de hospital y de la enfermedad porque se ha descrito incluso una prevalencia entre el 40-60% en los niños con enfermedad crónica. Con la idea de mejorar el cuidado nutricional en los niños la Sociedad Europea de Gastroenterología Hepatologíay Nutrición (ESPGHAN) ha recomendado el establecimiento de Unidades de Nutrición pediátrica que entre sus objetivos estarían la identificación de pacientes desnutridos y la instauración precoz de un adecuado soporte nutricional. Sin embargo en nuestro país hay muy pocas Unidades de Nutrición en los Hospitales Infantiles. En los últimos años se han desarrollado varios herramientas de cribado nutricional que son simples y útiles aunque han sido publicadas a partir de sus estudios locales sin haber sido probadas por varios autores. Es necesario que se compruebe su aplicabilidad, concordancia y utilidad para que puedan ser útiles. Es importante que las herramientas puedan ser incluidas en la práctica clínica para que puedan repercutir en el soporte nutricional precoz y adecuado (AU)

Malnutrition is associated to increased lenght of stay, more prescriptions and a higher economic cost and rate of infections. Iy is common in patients admitted to hospital. The prevention of malnutrition and hospital acquired malnutrition needs their early identification, included those patients who are undernourished and those with risk, but these recommendations have not been introduced in the rutine practice. Malnutrition in children has been associated with poor growth, reduced educational achievements and possible implications for adult health. The prevalence of acute malnutrition in infants and children ranges from 6.1% to 32% and in children who have underlying disease range increase (40-60%).In order to improve nutritional care in pediatrics the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) has recommended the stablishment of nutrition support teams, but in Spain this Teams are scarce now. Teams tasks should include identification of malnutriton risk patients and the provision of adequate nutritional support. In the last 10 years several simple and useful nutrition screening Tools for children have been developed. This Tools has been tested by their authors in their studies without validated in larger cohorts. It is also very important to include screening Tools as part as the standard pediatric care and to have the possibility to administer nutritional support to malnourished children (AU)